The objective is to evaluate the present status and future role of free vascular fibular transfer in treating congenital pseudoarthrosis of the tibia. [ncbi.nlm.nih.gov]

Reports described clinical presentation to range from simple anterolateral bowing of tibia to frank pseudarthrosis 3 .In this series 25 out of 37(60%) presented with established pseudarthrosis, while 10 out of 37(27%) presented with bowing. [sudjms.net]

Presenting problem: The table summarises patient characteristics, presenting problem, safeguarding assessment and action taken by social services. Café-au-lait lesions were present in all 3 patients. [bone-abstracts.org]

• Noncompliance

  […] significantly higher with a younger age at surgery (less than 4 years), low cross-sectional area of the healed segment, recurrence of tumor, when associated with persistent fibular pseudarthrosis, residual ankle valgus, removal of an intramedullary rod, and noncompliance [doi.org]

• Leg Swelling

  On clinical examination, the child was found to have multiple café-au-lait spots, axillary freckling, and right leg swelling [Figure 1]. [saudijhealthsci.org]

• Lymphedema

  […] tibia Congenital undergrowth of lower limb Congenital undergrowth of partial lower limb Congenital undergrowth of whole of lower limb Congenital valgus ankle Congenital varus ankle Constriction ring of lower limb Constriction ring of lower limb with lymphedema [icd9data.com]

• Joint Stiffness

  Clinically, the following parameters were assessed: pain, joint stiffness, and limb length discrepancy (LLD). [ncbi.nlm.nih.gov]

  Treatment is mainly surgical and it aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture. [scienceopen.com]

  Treatment is mainly surgical and it aims to obtain a long term bone union, to prevent limb length discrepancies, to avoid mechanical axis deviation, soft tissue lesions, nearby joint stiffness, and pathological fracture... [readbyqxmd.com]

• Recurrent Fractures

  However, surgical intervention is required in case of non-union and recurrent fractures. The surgical therapy consists of resection of the diseased segment and bone grafting, followed by internal or external fixation [ 3 ]. [e-aps.org]

The union rate was 94% with one treatment and 100% with two treatments. There were five refractures, three early and two late. Previous pin sites, residual angular deformity, and natural history were considered predisposing factors for refractures. [ncbi.nlm.nih.gov]

The authors followed up 9 cases of congenital pseudoarthrosis associated with neurofibromatosis-1 (or Von Recklinghausen's disease) at least 2 years after the end of treatment using Ilizarov's method. [moh-it.pure.elsevier.com]

The prognosis of CPT is very much related to the radiologic classification. Crawford type 2 and Crawford type 4 pseudoarthrosis have a worse prognosis, with a lower percentage of fusion at the site of pseudoarthrosis. [ncbi.nlm.nih.gov]

Prognosis continues to be reserved especially in clepsydra forms and those localised to the lower third of the tibia. [moh-it.pure.elsevier.com]

Pseudarthrosis develops through a congenital cyst Type IV : Pseudarthrosis develops in a sclerotic segment of bone without narrowing, stress fracture Type V : Pseudarthrosis of the tibia occurs with a dysplastic fibula, pseudarthrosis of one or both bones (prognosis [pseudarthrose.ch]

Type 2 have a relative poor prognosis. Type 3 cases are usually of late onset type which develops as a stress fracture of congenital dysplastic tibia or in a healed tibia after previous bone grafting & has the best prognosis. [de.slideshare.net]

Prognosis worse with Neurofibromatosis 1/2 of cases 3 Fracture develops at site of bone cyst Usually near junction of mid/ distal 1/3 Anterior bowing precedes or follow fracture 4 Sclerotic bony segment initially present at junction of mid/ distal 1/ [orthofracs.com]

Congenital pseudoarthrosis of the tibia (CPT) is an uncommon disease whose etiology and pathogenesis is unknown. [ncbi.nlm.nih.gov]

Pathology The etiology is unclear, however, around 50% of cases are associated with neurofibromatosis type 1 (NF1). Overall, 10% of patients with NF1 are diagnosed with tibial pseudoarthrosis. [radiopaedia.org]

Abstract The purpose of study was to refine frequency of etiological factors and characteristics of congenital pseudarthrosis of tibia (CPT). Materials and Methods. [journals.eco-vector.com]

Etiology Each type of tibial bowing tends to have its own etiology. [emedicine.medscape.com]

The etiological nature of this lesion is unclear, however there is a strong association between CPT and neurofibromatosis type I (NF-I). [jlponline.org]

Congenital pseudarthrosis of the tibia: history, etiology, classification, and epidemiologic data. J Pediatr Orthop B 2000; 9:11-15 [Google Scholar] 5. Grill F, Bollini G, Dungl P, et al. [ajronline.org]

Horn J, Steen H, Terjesen T (2013) Epidemiology and treatment outcome of congenital pseudarthrosis of the tibia. J Child Orthop 7(2):157–166 PubMedCentral CrossRef PubMed 20. [springermedizin.de]

Epidemiological data are limited, and treatment of the condition is challenging. The purpose of our study was to gain epidemiological data on the incidence of CPT in Norway and to evaluate the treatment outcome of the disease. [readbyqxmd.com]

Bowing usually at junction of Mid / Distal 1/3 Epidemiology Rare 1:200 000 M F 50% have Neurofibromatosis type 1 1-3% of Neurofibromatosis have CPT Aetiology Cause unknown Theories include: Intrauterine fracture Localised vascular abnormality Constriction [orthofracs.com]

Congenital pseudoarthrosis of the tibia: History, etiology, classification and epidemiologic data. J Pediatr Orthop B 2000;9:11-5. [ PUBMED ] 2. Andersen KS, Bohr H, Sheppen O. Congenital angulation of the lower leg. Crus curvatum congenitum. [jlponline.org]

Pathophysiology, evaluation, and treatment. J Bone Joint Surg Am 2005;87:1848-64. Wang K, Allen L, Fung E, et al. Bone scintigraphy in common tumors with osteolytic components. Clin Nucl Med 2005;30:655-71. Tuncel M, Kiratli PO, Gedikoglu G. [journals.sfu.ca]

A relationship with type 1 neurofibromatosis or von Recklinghausen’s disease is known but exact pathophysiology has still not been clearly defined. [boneandspine.com]

Appropriate interpretation will improve understanding of the natural history and pathophysiology of neurofibromatosis type 1. [nature.com]

The pathophysiology of the resultant limb-length inequality may be related to the bowing. [emedicine.medscape.com]

At the end of the correction, a telescopic Fassier-Duval intramedullary nail was used prophylactically to prevent re-fracture. After 2 years of follow-up, X-ray images show bone-healing without any bowing. [ncbi.nlm.nih.gov]

Moreover it does not always prevent ankle valgus [Figure 4]. To prevent all these, the rod must be kept till skeletal maturity. [doi.org]

Created to prevent or stabilize fractures, or correct deformity of long bones whilst growth occurs. [pegamedical.com]

poor protection against re-fracture, failure to correct limb length discrepancy, and deformities of leg and ankle simultaneously at time of primary surgery. 8 Vascularized fibular graft/ Nail The intramedullary rod offers good tibial alignment and prevents [slideplayer.com]